1. Dr.Emamzadegan Pediatric & Congenital Cardiologist
Cyanotic CHD
Dr.Emamzadegan
Pediatric & Congenital Cardiologist

2. Cyanotic CHD # Etiology : Rt to Lt shunt
# Cyanosis: o2 sat < 85% ,5 g/dl of reduced Hgb in systemic blood
# Anemia: decreased cyanosis
Polycythemia: increased cyanosis
#The most common type:5 T(TOF;Truncus arteriosus;d-TGA;Tricuspid Atresia;TAPVC)

3. Cyanotic CHD # Hypoperfusion: # Cyanosis with respir. distress:
increased pulm. blood flow (d-TGA)
# Cyanosis without respir. distress:
decreased pulm. blood flow (TOF)
# Hypoperfusion:
TAPVC with obstruction;AS;HLHS;CMP

4. Tetralogy Of Fallot # The most common cyanotic CHD(10%)
# PS(the most common: infundibular) ; VSD (sub Ao); Ao overriding ; RVH(secondary)

5. Tetralogy Of Fallot Clinical Manifestation:
# degree of cyanosis: amount of PS
# Initial MM: PS (upper LSB)
#S2 : Single ,RV impulse in LSB
#Hypoxic spell (blue spell, tet spell , hypercyanotic spell):
restless, agitated, loss of murmur , hyperpnea, unconsciousness , seizure, hemiparesia , death

6. Tetralogy Of Fallot # ECG: RAD,RVH # CXR: boot shaped heart ,PVM↓
# Complications: brain abscess ;cerebral thromboembolism
# ECG: RAD,RVH
# CXR: boot shaped heart ,PVM↓

7. Tetralogy Of Fallot Treatment:
Spell: Knee chest position, O2, morphine , phenylephrine, Inderal, SBE prophylaxis,…….TFTC(5% LAD from RCA, crossing RVOT)
Blalock-Taussig shunt (subclavian to PA)

8. D-TGA # Mixing : atrial,ventricular,PDA # cyanosis: amount of mixing
# 5% of CHD
# The most common cyanotic CHD to present in the newborn period: d-TGA
# Mixing : atrial,ventricular,PDA
# cyanosis: amount of mixing
#S2: single(A2)

9. D-TGA # d-TGA/IVS:no MM, severe cyanosis
d-TGA/VSD: mild cyanosis ,HF, loud mm
# ECG: RAD,RVH
# CXR: PVM↑ , egg on string
#Treatment: PGE1,Rashkind balloon septostomy,surgery of choice (Arterial switch) within 2 weeks of life.

10. Tricuspid Atresia # 2% of CHD
# Hypoplastic RV, Rt to Lt shunt at ASD level, VSD is necessary
# Severe cyanosis, single S2,frequently no significant MM( VSD MM, diastolic MM of MV)

11. Tricuspid Atresia # ECG: LVH, superior axis(0 to -90)LAD PVM ↓
# CXR: NL size of heart or mild ↑
PVM ↓
# Treatment:
Shunt (Blalock-Taussig): subclavian to PA
Glenn (SVC to RPA)
Fontan (IVC to PA)

12. Truncus Arteriosus
# Single arterial trunk arises from heart with large VSD, pulmonary arteries arise from trunk.
# Cyanosis: amount of pulm. Flow
# CHF: tachypnea , cough
# Pulses: bounding
# Heart: S2= single ,SMM(LSB), systolic ejection click

13. # treatment: Digoxin +diuretic>>>>surgery
Truncus Arteriosus
ECG : CVH
CXR: PVM↑,PA may appear displaced
# treatment: Digoxin +diuretic>>>>surgery

14. TAPVC # Disruption of development of PV during 3 week of gestation.
# Typing: supracardiac, cardiac,infra, mixed
# ASD with Rt to Lt shunt is necessary

15. TAPVC Clinical manifestations: 2.With obstruction
1.Without obstruction
2.With obstruction

16. 1.Without obstruction ECG: RVH,RAD CXR: C/T & PVM ↑
Mild cyanosis, may be asymptomatic, continuous murmur, wide s2 split, systolic ejection MM (upper LSB)
ECG: RVH,RAD
CXR: C/T & PVM ↑

17. 2.With obstruction ECG: RAD,RVH CXR: C/T= Normal,
Cyanosis, tachypnea, dyspnea, Rt heart failure (hepatomegaly), no MM
ECG: RAD,RVH
CXR: C/T= Normal,
pulmonary edema (HMD, pneumonia)

18. HLHS # The most common cause of death during neonatal period from CHD.
#Hypoplastic LV, hypoplasia of ascending aorta& Ao arch.
# Lt to Rt shunt at ASD level,Rt to Lt shunt at PDA level, retrograde flow in AAO.

19. HLHS Clinical manifestations: If PDA constricts: CHF & death
pulses=weak,
S2 =single & loud
No MM, Mild cyanosis
Grayish color, cool & mottled skin
If PDA constricts: CHF & death

20. HLHS ECG: RVH CXR: C/T ↑ ,pulmonary edema Treatment:
PGE1,correction of acidosis, vent.support, >>> Norwood ,Glenn, Fontan

21. Extracardiac complications of cyanotic CHD
1.Polycythemia
2. Anemia
3.CNS abscess & emboli
4.Gingival disease
5.Gout(polycythemia & diuretic)
6.Clubbing(hypoxia)
7.Infectious disease (asplenia;Di George;RSV)
8.FTT